Hermaszewski R A, Webster A D
Clinical Research Centre, Harrow.
Q J Med. 1993 Jan;86(1):31-42.
The records of 240 patients with common variable immunodeficiency, seven with thymoma-associated hypogammaglobulinaemia and 44 patients with X-linked agammaglobulinaemia seen at this centre over the past 20 years, were reviewed. Although substantial advances have been made in treatment there continues to be a delay in diagnosis, as well as high mortality and morbidity rates. Hypogammaglobulinaemia is associated with a high incidence of chronic sinopulmonary infection, chronic diarrhoea, malignancy, joint disease and hepatitis. There is particular concern that infection with mycoplasmas and enteroviruses can be resistant to treatment. The high incidence of lymphoma and gastric carcinoma in patients with common variable immunodeficiency is high-lighted.
回顾了本中心在过去20年中诊治的240例普通可变型免疫缺陷患者、7例胸腺瘤相关低丙种球蛋白血症患者和44例X连锁无丙种球蛋白血症患者的病历。尽管在治疗方面已取得了重大进展，但诊断仍存在延误，死亡率和发病率也居高不下。低丙种球蛋白血症与慢性鼻窦肺部感染、慢性腹泻、恶性肿瘤、关节疾病和肝炎的高发病率相关。特别令人担忧的是，支原体和肠道病毒感染可能对治疗产生耐药性。普通可变型免疫缺陷患者中淋巴瘤和胃癌的高发病率值得关注。
